Connective tissue disease

2019 ◽  
pp. 281-326
Author(s):  
Gavin Spickett
Keyword(s):  
Rheumatoid Arthritis ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Mixed Connective Tissue Disease ◽  
Raynaud’S Phenomenon ◽  
Connective Tissue Diseases ◽  
Raynaud's Phenomenon ◽  
Overlap Syndromes

This chapter covers the presentation, immunogenetics, immunopathology, diagnosis, treatment, and testing for a range of connective tissue diseases. It covers a range of rheumatic disorders, from rheumatoid arthritis to Raynaud’s phenomenon, and also covers the undifferentiated diseases, overlap syndromes, and mixed connective tissue disease.

Overlap/undifferentiated syndromes

2013 ◽  
pp. 1069-1078 ◽  
Author(s):  
Ariane Herrick
Keyword(s):  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Clinical Features ◽  
Lupus Erythematosus ◽  
Raynaud’S Phenomenon ◽  
Clinical Manifestations ◽  
Raynaud's Phenomenon ◽  
Overlap Syndromes ◽  
Clear Cut ◽  
Positive Treatment

Undifferentiated connective tissue disease (UCTD) and overlap syndromes both form part of the broad spectrum of connective tissue disease. They are difficult to define, as the boundaries between them and specific diseases such as systemic lupus erythematosus (SLE), systemic sclerosis (SSc), and myositis are often not clear-cut. This chapter gives a broad overview of diagnosis, clinical features, outcomes, and management. Patients with UCTD have clinical and/or serological features of connective tissue disease but do not fulfil the criteria for any one defined disease. Raynaud’s phenomenon and puffy fingers are often the presenting features but there are many possible others, including arthralgia, sicca symptoms, and breathlessness due to pulmonary fibrosis, usually in the context of a positive anti-nuclear antibody (ANA). A proportion of patients evolve into a defined connective tissue disease: in those who do, this is generally within 5 years of onset. Treatment is dependent upon the clinical features: for examplem vasodilators for Raynaud’s phenomenon, or hydroxychloroquine for arthralgia/arthritis. Patients with overlap syndromes have features of more than one defined connective tissue disease. Overlap syndromes are therefore highly heterogeneous as many combinations of clinical and serological features can occur. Mixed connective tissue disease (MCTD) is the overlap syndrome that has been most described and includes overlapping features of SLE, SSc, and myositis in patients who are anti-U1 ribonucleoprotein (RNP) antibody positive. Treatment is of the specific clinical manifestations. Patients with overlap syndromes should be kept under regular review to allow early identification of internal organ involvement.

scholarly journals Relationship between antibodies against extractable antigens from the nucleus and connective tissue diseases identified by Immunoblots in a hospital and university in Lima

2020 ◽  
pp. 25-30
Author(s):  
José Enrique Oliva Menacho ◽  
Jorge Luis Arroyo Acevedo ◽  
Jose Arturo Oliva Candela ◽  
Percy Genaro Salas Ponce ◽  
Marco Antonio Garcia Hjarles
Keyword(s):  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Significant Relationship ◽  
Raynaud’S Phenomenon ◽  
Connective Tissue Diseases ◽  
Raynaud's Phenomenon ◽  
Esophageal Dysmotility ◽  
Chi Square ◽  
Chi Square Test ◽  
Relationship Of

Objectives: To determine the relationship of antibodies to extractable nucleus antigens and connective tissue diseases identified by Immunoblot in a hospital in Lima, Peru. Material and methods: Study of the observational type, basic sciences, analytical and trans-versal, carried out in the Immunology service of the national Hospital Archbishop Loayza between January 2018 and June 2018. We analyzed 291 clinical histories of patients with connective tissue disease and for the detection of antibodies to the extractable antigens of the nucleus the method of Immunoblot was employed. Results: The frequency of the antibodies against extractable nuclear antigens in patients with connective tissue disease identified by Immunoblot was 789 (100%). It was demonstrated that there is significant relationship p < 0.05 of Anti-histones (X2 = 64.19; p = 0,000), an-ti-nucleosomas (X2 = 71,16; p = 0,000), anti-dsDNA (X2 = 71,44; p = 0,000), anti-SM (X2 = 10,08; p = 0,003) and Lupus Systemic erythematosus with Pearson Chi-square test. It was demons-trated that there is significant relationship p < 0.05 of the Anti-SSA (X2 = 61,33; p = 0.001), anti-SSB (x2 = 51,00; p = 0.001), anti-Ro 52 (X2 = 62,60; p = 0,000) and Sjogren’s syndrome with Pearson Chi-square test. It was demonstrated that there is significant relationship p < 0.05 of Anti-CENP B (p = 0.001) and calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly and Telangiectasia (CREST) with exact Fisher statistician. Conclusions: There is a relationship of antibodies to extractable nucleus antigens and systemic lupus erythematosus, Sjogren’s syndrome, mixed connective tissue disease, calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly and Telangiectasias (CREST), Scleroderma and Polymyositis.

Overlap/undifferentiated syndromes

2013 ◽  
pp. 1069-1078
Author(s):  
Ariane Herrick ◽  
Michael Hughes
Keyword(s):  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Clinical Features ◽  
Lupus Erythematosus ◽  
Raynaud’S Phenomenon ◽  
Clinical Manifestations ◽  
Raynaud's Phenomenon ◽  
Overlap Syndromes ◽  
Clear Cut ◽  
Positive Treatment

Undifferentiated connective tissue disease (UCTD) and overlap syndromes both form part of the broad spectrum of connective tissue disease. They are difficult to define, as the boundaries between them and specific diseases such as systemic lupus erythematosus (SLE), systemic sclerosis (SSc), and myositis are often not clear-cut. This chapter gives a broad overview of diagnosis, clinical features, outcomes, and management. Patients with UCTD have clinical and/or serological features of connective tissue disease but do not fulfil the criteria for any one defined disease. Raynaud’s phenomenon and puffy fingers are often the presenting features but there are many possible others, including arthralgia, sicca symptoms, and breathlessness due to pulmonary fibrosis, usually in the context of a positive anti-nuclear antibody (ANA). A proportion of patients evolve into a defined connective tissue disease: in those who do, this is generally within 5 years of onset. Treatment is dependent upon the clinical features: for example vasodilators for Raynaud’s phenomenon, or hydroxychloroquine for arthralgia/arthritis. Patients with overlap syndromes have features of more than one defined connective tissue disease. Overlap syndromes are therefore highly heterogeneous as many combinations of clinical and serological features can occur. Mixed connective tissue disease (MCTD) is the overlap syndrome that has been most described and includes overlapping features of SLE, SSc, and myositis in patients who are anti-U1 ribonucleoprotein (RNP) antibody positive. Treatment is of the specific clinical manifestations. Patients with overlap syndromes should be kept under regular review to allow early identification of internal organ involvement.

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